Amendment of the OMERACT ultrasound definitions of joints' features in healthy children when using the DOPPLER technique.

BACKGROUND: Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler findings in the evaluation and definition of normal joint features that can be visualized in healthy children at different age groups. METHODS: A multistep approach was used. Firstly, new additional definitions of joint components were proposed during an expert meeting. In the second step, these definitions, along with the preliminary B-mode-US definitions, were tested for feasibility in an exercise in healthy children at different age groups. In the last step, a larger panel of US experts were invited to join a web-based consensus process in order to approve the developed definitions using the Delphi methodology. A Likert scale of 1-5 was used to assess agreement. RESULTS: Physiological vascularity and fat pad tissue were identified and tested as two additional joint components in healthy children. Since physiological vascularity changes over the time in the growing skeleton, the final definition of Doppler findings comprised separate statements instead of a single full definition. A total of seven statements was developed and included in a written Delphi questionnaire to define and validate the new components. The final definitions for fat pad and physiological vascularity agreed by the group of experts reached 92.9% and 100% agreement respectively in a web survey. CONCLUSION: The inclusion of these two additional joints components which are linked to detection of Doppler signal in pediatric healthy joints will improve the identification of abnormalities in children with joint pathologies.

Juvenile, adult and late-onset systemic lupus erythematosus: a long term follow-up study from a geographic and ethnically homogeneous population.

OBJECTIVES: This paper aims to identify clinical and serological differences, damage accrual and mortality, in juvenile, adult and late-onset SLE. METHODS: We conducted our study with patients fulfilling SLE classification criteria taken from the Hospital Gregorio Marañon Autoimmune Systemic Rheumatic Diseases' Registry (1986 to 2012). Clinical characteristics, laboratory data and therapies used during the course of the disease were analysed with patients divided into 3 groups: juvenile-onset (≤ 18 years), adult-onset (19-50) and late onset (>50 years). RESULTS: Four hundred and forty-five patients were included. Renal disease and cutaneous manifestations were more frequent in the juvenile-onset group at disease onset. During follow-up, juvenile-onset group presented a higher incidence of renal disease, malar rash, Raynaud's phenomenon, cutaneous vasculitis, and neuropsychiatric manifestations than the other two groups. Arthritis and lymphopoenia were more frequent in the adult-onset group. Arterial hypertension and neoplasm were more frequent in the late-onset group. Low serum complement, anti-dsDNA, anti-U1RNP and anti-Sm antibodies were more common in the juvenile-onset group. Patients with late-onset SLE had more damage accrual. Thirty-seven patients (8.3%) died during the study. All-cause mortality was significantly higher in the late-onset group. Age at disease onset >50 years was an independent risk factor for damage accrual (OR, 2.2; 95%CI, 1.1-4.6; p=0.029) and mortality (OR, 2.6; 95%CI, 1.1-6.3; p=0.03). CONCLUSIONS: We found significant differences in clinical and serological profiles between juvenile, adult and late-onset SLE. The most significant of which was a higher prevalence of neuropsychiatric and renal complications as well as different autoantibody signatures for the juvenile-onset group.

Investigating the link between disease activity and infliximab serum levels in rheumatoid arthritis patients.

OBJECTIVES: The aim of this study was to examine the extent to which infliximab (IFX) serum levels impact disease activity in rheumatoid arthritis (RA) patients. METHODS: In this cross sectional study, serum samples were taken prior to drug infusion from 60 RA patients who had been undergoing IFX therapy > 12 months as a first line of biological treatment. Patient IFX levels were tested and then associated with clinical disease activity. Three DAS28 cut-off points, <2.6, <3.2 and <5.1 were used to determine whether detectable IFX levels were any predictor of clinical disease activity. Logistic regression analysis was run to check other possible factors associated with RA clinical outcomes such as MTX concomitant use, CRP and ESR. RESULTS: Sixteen (27%) out of the 60 patients tested negative; 28 (46%) presented subtherapeutic and 16 (27%) therapeutic IFX levels. Median IFX levels were higher in patients either in remission or showing low disease activity than in those with moderate and high disease activity (p=0.014). Significant association was found between IFX levels and clinical disease activity (p=0.001). Detectable levels of IFX shows better sensitivity and specificity to identify patients with DAS28<3.2 than to identify patients with DAS28<2.6 or DAS28<5.1. Conversely, the best DAS28 cut-off to identify detectable/undetectable IFX was 3.19, with AUC under ROC curve 0.804 (Sd.E 0.070), 76% specificity and 83% sensitivity (p<0.001). MTX use, CRP and ESR did not interfere with this association. Seven out of the 8 patients with anti-IFX antibodies presented DAS28>3.2 (p=0.005). CONCLUSIONS: DAS28 and IFX serum levels were shown to have an inverse correlation. Undetectable IFX serum levels were associated to RA patients presenting DAS28>3.2 meaning that DAS28 <3.2 may be useful to clinicians to evaluate patient response to drug therapy.

Agreement of non-contact pachymetry after LASIK: comparison of combined scanning-slit/Placido disc topography and specular microscopy.

PURPOSE: To assess the interchangeability of central corneal thickness (CCT) measurements between combined scanning-slit/Placido disc topography (Orbscan-II) and specular microscopy (Topcon SP-2000P) in patients who underwent laser in situ keratomileusis (LASIK) for myopia. PATIENTS AND METHODS: We recruited 118 consecutive patients who underwent LASIK for myopia. The CCT was measured using Orbscan-II and Topcon SP-2000P randomly. Orbscan-II and Topcon SP-2000P CCT data were analysed using the paired-sample t-test and the limits of agreement (LoA) were calculated with the method described by Bland-Altman. RESULTS: The average CCT measurements by Orbscan-II and Topcon SP-2000P were 447.55+/-49.78 microm and 461.38+/-35.35 microm, respectively (P<0.0001; mean difference, 13.83+/-22.31 microm; 95% confidence interval, 9.77-17.09 microm). The Bland-Altman plot showed an inverse association between the average and the difference between the devices: Topcon-Orbscan-II=174.23 -0.353xAverage (P<0.01). The widths of the crude and regression-based 95% limits of agreement were 87.45 and 63.72 microm, respectively. CONCLUSIONS: Orbscan-II measurements of CCT after myopic LASIK were significantly lower than those obtained using Topcon SP-2000P. The limits of agreement between the two devices were too broad and, therefore, both pachymetric values cannot be used interchangeably. Further, the tendency towards comparably Orbscan-II readings in thinner corneas precludes that one technique can directly replace the other. This is important for the adequate medium- and long-term follow-up of the growing LASIK patient population.

Primary trochlear headache: a new cephalgia generated and modulated on the trochlear region.

BACKGROUND: The authors have observed a group of patients complaining of periorbital pain, emanating from the trochlear area, in absence of trochleitis or other orbital or systemic disease. All were previously diagnosed and treated as different types of headaches, but pain was not controlled until local treatment on the sore trochlea was performed. The authors have investigated the role of the trochlear area in causing and modulating headache. METHODS: Observational case series. Trochlear pain was defined as pain on this area, exacerbated upon examination and looking in supraduction. Pain was studied after trochlear injections of lidocaine, corticosteroids, and placebo. Secondary orbital pain was ruled out. RESULTS: Seventeen women and one man were evaluated (mean age: 44 years). All presented unilateral pain in the trochlear area (60% reported more extended headache), for more than 1 year in 70%. Neither ocular autonomic signs nor motility restrictions were observed. Imaging examinations were normal in 100%. The temporal pattern was either chronic or remitting, with acute exacerbations. Pain increased at night in 55%. A total of 62% presented concurrent headaches. Locally injected corticosteroids relieved the pain within 48 hours in 95% and also improved concurrent headaches, by decreasing attack frequency and analgesics intake. Placebo was not helpful. Relapses were observed in 45% (average 8 months). CONCLUSIONS: The trochlear region is the origin of a specific and unrecognized headache, which we have named primary trochlear headache. Local treatment on the trochlear area is also useful for other concurrent primary headaches with inadequate response to oral therapy.

Pulmonary mass and multiple lung nodules mimicking a lung neoplasm as amiodarone-induced pulmonary toxicity.

Amiodarone is an effective anti-arrhythmic agent. However, during long-term therapy, patients can develop severe adverse pulmonary reactions that are potentially life-threatening. A case of amiodarone-induced pulmonary toxicity is presented in a 78-year-old woman. She developed dyspnea and a pulmonary mass with associated multiple lung nodules mimicking a lung cancer following 5 years of treatment with amiodarone for atrial fibrillation. After drug withdrawal, and without any additional treatment, clinical and radiological improvement was observed, and radiological findings resolved completely within 6 months.

Depressive disorders in epilepsy.

Depression is a common occurrence among epileptic patients and constitutes, along with anxiety disorders, the most frequent psychiatric condition in these patients. The relationship between depression and epilepsy is two-directional, because patients with major depression also have a higher frequency of epilepsy. In epileptic patients, depressive disorders can present as unipolar, bipolar, or dysthymic disorders. More characteristically, however, they present as an atypical depression, which can often go unrecognized for long periods of time. In the diagnostic evaluation of these patients, clinicians must rule out the possibility that the depressive disorder resulted from the administration of antiepileptic drugs (AEDs; e.g., barbiturates) or from the discontinuation of an AED with mood-stabilizing properties that were masking an underlying affective disorder. Although antidepressant drugs have been used in epileptic patients for a long time, to date there has only been one controlled study. The antidepressants of the family of selective serotonin reuptake inhibitors (SSRIs) should be considered as initial therapy for depressive disorders in these patients.

[Pulmonary artery aneurysm. A case report]. / Aneurisma de la arteria pulmonar. Presentación de un caso.

The pulmonary artery aneurysm is a rare clinical entity that presents a low incidence and prevalence, of difficult diagnosis to be presented with poorly specific symptoms or also without symptoms, being detected in radiological studies as a widening or mediastinal mass. It can be uni or bilateral and presenting itself isolated or in the context of other sicknesses. The diagnosis of certainty is based in the realization of Echo-Doppler and other studies as a tomography or a magnetic resonance, the therapeutic option being so difficult, and according to cases, by an expectant or aggressive attitude.

Characterization and selection of lactobacilli isolated from Spanish fermented sausages.

The use of starter cultures to control and run the fermentative process is a usual way of manufacturing sausages in meat industries. The first stage in the starter culture designing process is to characterize the lactic acid bacteria isolated from these meat products, in order to select the best strains. The strains used for this study were isolated from different dry fermented sausages, obtained during the manufacturing process. The main tests used to identify the isolated bacteria were: microscopic-morphologic characteristics, catalase activity, production of gas, growth at 8, 15 and 45 degrees C, fermentation of carbohydrates and production of lactic acid isomers. A total of 194 strains were identified. Lactobacillus sake and Lactobacillus plantarum were the most frequent species. Other microbiological tests were also performed, and three strains of Lactobacillus sake were found which did not produce dextran from sucrose.

[Increased levels of erythropoietin in the initial phase of acute myocardial infarction]. / Elevación de la eritropoyetina en la fase inicial del infarto agudo de miocardio.

BACKGROUND: It's know that cardiopulmonary function affects the kidney perfusion and that erythropoietin (EPO) release depends on it. We want to determine the plasma level of EPO in the acute phase of myocardial infarction (AMI). SUBJECTS AND METHODS: A transversal trial was carried out in 37 male patients with AMI aged between 31 and 84. We studied the following variables: cardiovascular risk factors, time lapse from beginning of symptoms until hospital arrival, transcutaneous oxygen saturation (ST02) and EPO plasma levels. 16 healthy males were used as control group. RESULTS: Patients with AMI have different EPO levels than control group (25/37 vs 0/16) (18.90 +/- 8.43 mUI/ml vs 9.70 +/- 3.48 mUI/ml respectively p < 0.001). Hyperintense patients have higher EPO levels than normotense ones (18.53 +/- 8.28 mUI/ml vs 12.88 +/- 7.29 mUI/ml p < 0.05). Hypercholesterolemic patients have higher EPO level than normocholesterolemic ones (19 +/- 8.88 mUI/ml vs 12.40 +/- 6.75 mUI/ml p < 0.01). There were no difference between smokers and no smokers. We didn't find correlation between time lapse and EPO levels. CONCLUSION: The trial remarks EPO levels increase during the initial phase of AMI and it is higher in hypertensive and hypercholesterolemic patients.

[The Holt-Oram syndrome. Entelechy or clinical entity? A mild form of familial presentation]. / Síndrome de Holt-Oram. Entelequia o entidad clínica? Una forma leve de presentación familiar.

According to the description made by Holt and Oram in 1960, a wide group of associated skeletal and cardiac malformations was named as syndrome of Holt-Oram. The morpho-anatomic and functional expression of the syndrome are so great in extension, that it is possible to think of several clinical entities with common manifestations; or perhaps, in a common entity with different clinical and functional expressions. Undoubtedly, the interest in the knowledge and diagnostic of the disease is due mainly to the hereditary condition, as demonstrated in a great number of cases, though they had been observed some generations without antecedents, with the possible explanation of genetic mutation. In this paper, are demonstrated the descriptive findings in a patient, male, 32 years old, with the diagnostic of Holt-Oram syndrome, and also the results of the study of the near members of his family. This study allowed to find other affected persons with manifestations that probably can be the result of new mutations. The paper is completed with a wide review of the pertinent bibliography.